muscle disease(mg vs myopathy ocular perspective)
TRANSCRIPT
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Myasthenia and Myopathies -
An Ocular Perspective.
Dr. V. Natarajan.
Additional Professor of NeurologyInstitute of NeurologyGovernment General Hospital and Madras Medical College
Presentation atCME on Neuro-ophthalmic Disorders.
at Regional Institute of Ophthalmology andGovernment Ophthalmic Hospital, Chennai.
September 16, 2006.
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MYASTHENIA
Ptosis or Diplopia
Initial symptom in 2/3
Fluctuation
Spontaneous improvement
Restricted weakness 10-40% Progression in 2 years
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Findings
One or more ocular muscle
Pupils spared
Variable weakness, fatigability
Shift of ptosis Pathognomonic
Ocular quiver Superfast saccades
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Lid Twitch
Lifting ptotic lid may cause the other lid to
fall
Covering of ptotic lid relieves contraction
of opposite frontalis
Ice pack test
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Investigations
Anti Ach R antibody titre
False positive is rare
50% of OMG Antibody negative
15% of GMG Antibody negative
Anti Musk Ab 50% sero negative Ach R
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Edrophonium (Tensilon) Test
Superseded
Small risk of respiratory arrest, cardiac
arrhythmias
Resuscitation facilities
Pre dose with Atropine
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Ideal dose cannot be predetermined
escalating dose 2mg, 3mg, 5mg IV
IM Neostigmine
Positive in MND and OMN lesions
Negative test does not exclude MG
Edrophonium (Tensilon) Test
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Neurophysiologic Tests
RNS
Supra maximal 3 Hz decrement
SF-EMG More sensitive
2 muscle fibers in 1 unit
Inter potential interval variability
Jitter / Block
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Treatment
Ach EI Symptom relief
Steroids Individualized
Thymectomy Debatable
Young patients
Recent onset Poor response to Ch EI
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OCULO PHARYNGEAL
MUSCULAR DYSTROPHY
Same age group as MG 5th & 6th decade
EOM & Ptosis asymmetrical
Swallowing difficulty
Facial & limb weakness
EMG & Muscle biopsy
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Myotonic Dystrophy
Ptosis, no EOM involvement
Facial appearance wasting
Associated features Cardiac
Cataract
Endocrine & Testicular atrophy
Myotonia on percussion
EMG
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Mitochondrial myopathy
Progressive external ophthalmoplegia with
or without limb weakness
RP, heart block, endocrinopathies Lactate, Pyruvate
CSF proteins
Muscle biopsy
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Congenital Myopathies
Centro muscular or myotubular myopathy
X linked recessive- infantile; AD- in later
life
Ptosis, EOM, facial and limb muscles
EMG Spontaneous activity
Muscle biopsy
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Tolosa Hunt Syndrome
Inflammatory pathology in anterior
cavernous sinus, SOF
Usually painful
EOM paresis, asymmetrical
Steroid responsive
Recurrent Associated V, II helpful
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Partial III Nerve
Superior branch
Ptosis, Superior rectus
Pupil spared
Idiopathic, viral, aneurysm of IC, PCom
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Congenital Myasthenic Syndromes (CMS)
Heterogenous group
Genetic defects affecting NMT
Presynaptic, synaptic and post synaptic
defects
Muscle weakness Increases by exertion
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Early onset
Opthalmoplegia, ptosis, bulbar
Amyotrophy, tendinous retraction facial
malformation
Family history
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